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Management and outcomes of hypertrophic cardiomyopathy in young adults
Émilie Baron1, Nicole Karam2, Erwan Donal3
1Cardiology department, Hôpital européen Georges Pompidou, AP-HP, 75015 Paris, France.
Insights
Major adverse cardiac events (MACE) are common in young adults with hypertrophic cardiomyopathy (HCM), particularly in obstructive cases and women. Current guidelines for implantable cardioverter defibrillator (ICD) use are imperfect for identifying sudden cardiac death (SCD) risk.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) management in young adults presents significant clinical challenges.
- Young adults with HCM require specific evaluation due to unique risk profiles and long-term implications.
Purpose of the Study:
- To characterize the clinical profile of young adults (16-25 years) diagnosed with HCM.
- To evaluate the incidence and nature of major adverse cardiac events (MACE) in this cohort.
- To assess the adherence to and effectiveness of current guidelines for risk stratification and management.
Main Methods:
- Prospective registry analysis of young adults (16-25 years) with HCM.
- Comparison of patients based on the occurrence of MACE, including sudden cardiac death (SCD) events, atrial fibrillation/embolic stroke, heart failure hospitalization, and syncope.
- Follow-up duration averaged 4.4±2.2 years, with baseline and follow-up data analyzed for risk factors and outcomes.
Main Results:
- At baseline, 21.3% had prophylactic ICDs, 24.6% had a family history of SCD, and 29.5% had obstruction. Myocardial fibrosis was present in 86.0%.
- During follow-up, 24.6% of patients experienced MACE, primarily SCD events (41.2%) and atrial fibrillation/stroke (35.3%).
- Obstructive HCM significantly increased MACE risk (OR 3.96, P=0.035). ICD implantation rates did not consistently follow guidelines, especially in high-risk patients (score ≥6%).
Conclusions:
- MACE are frequent in young adults with HCM, especially in obstructive forms and women, with arrhythmic events predominating.
- Prophylactic ICD implantation is common but not always guideline-adherent, indicating a need for refined risk assessment tools.
- Existing European/USA guidelines aid in identifying SCD risk but are imperfect, highlighting the need for improved predictive models in this population.
Background:
Management of young adults with hypertrophic cardiomyopathy (HCM) is challenging.
Aims:
To evaluate the profile of young adults (16-25 years) with HCM included in the French prospective HCM registry.
Methods:
Patients were compared according to occurrence of major adverse cardiac events (MACE), comprising sudden cardiac death (SCD) events (implantable cardioverter defibrillator [ICD] discharge, SCD, sustained ventricular tachycardia), atrial fibrillation/embolic stroke, heart failure hospitalisation and unexplained syncope, at a mean follow-up of 4.4±2.2 years.
Results:
At baseline, among 61 patients (20.5±3.0 years; 16 women, 26.2%), 13 (21.3%) had a prophylactic ICD, 24.6% a family history of SCD, 29.5% obstruction, 86.0% magnetic resonance imaging myocardial fibrosis, 11.8% abnormal exercise blood pressure and 52.8% a European Society of Cardiology (ESC) 5-year SCD score<4% (24.5%≥6%). At follow-up, 15 patients (24.6%; seven women; all with fibrosis) presented 17 MACE, comprising: SCD events (n=7, 41.2%; including three patients with an ICD, five with at least one SCD major classical risk factor and an ESC score≥5% and two with no risk factors and an ESC score<4%); atrial fibrillation/stroke (n=6, 35.3%); heart failure (n=1, 5.9%); syncope (n=3, 17.6%). An ICD was implanted in 11 patients (four for secondary prevention), but in only 61.5% of patients with a score≥6%. Only obstruction significantly increased MACE risk (odds ratio 3.96; P=0.035), with a non-significant trend towards a lower risk in men (OR 0.29; P=0.065).
Conclusions:
In young adults with HCM, MACE are common in the short term, especially in obstructive HCM and women, mostly arrhythmic in origin. Prophylactic ICD implantation is frequent and does not strictly follow the guidelines, while the use of European/USA guidelines is helpful but imperfect in identifying SCD risk.
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